Zheng Yongzhi, Huang Yan, Le Shaohua, Zheng Hao, Hua Xueling, Chen Zaisheng, Feng Xiaoqin, Li Chunfu, Zheng Mincui, Xu Honggui, He Yingyi, He Xiangling, Li Jian, Hu Jianda
Department of Hematology, Fujian Institute of Hematology, Fujian Provincial Key Laboratory on Hematology, Fujian Medical University Union Hospital, Fuzhou, China.
Department of Pediatrics, Southern Medical University/Nanfang Hospital, Guangzhou, China.
Front Oncol. 2021 Sep 13;11:712747. doi: 10.3389/fonc.2021.712747. eCollection 2021.
A high ecotropic viral integration site 1 ( expression ( ) is an independent prognostic factor in adult acute myeloid leukemia (AML). However, little is known of the prognostic value of in pediatric AML. This study aimed to examine the biological and prognostic significance of in uniformly treated pediatric patients with AML from a large cohort of seven centers in China.
A diagnostic assay was developed to determine the relative expression using a single real-time quantitative polymerase chain reaction in 421 newly diagnosed pediatric AML patients younger than 14 years from seven centers in southern China. All patients were treated with a uniform protocol, but only 383 patients were evaluated for their treatment response. The survival data were included in the subsequent analysis (n = 35 for , n = 348 for ).
was found in 9.0% of all 421 pediatric patients with AML. was predominantly found in acute megakaryoblastic leukemia (FAB M7), rearrangements, and unfavorable cytogenetic aberrance, whereas it was mutually exclusive with t (8; 21), inv (16)/t (16; 16), , , or mutations. In the univariate Cox regression analysis, had a significantly adverse 5-year event-free survival (EFS) and overall survival (OS) [hazard ratio (HR) = 1.821 and 2.401, = 0.036 and 0.005, respectively]. In the multivariate Cox regression analysis, was an independent prognostic factor for the OS (HR = 2.447, = 0.015) but not EFS (HR = 1.556, p = 0.174). Furthermore, was an independent adverse predictor of the OS and EFS of patients with rearrangements (univariate analysis: HR = 9.921 and 7.253, both < 0.001; multivariate analysis: HR = 7.186 and 7.315, = 0.005 and 0.001, respectively). Hematopoietic stem cell transplantation (HSCT) in first complete remission (CR1) provided patients with a tendential survival benefit when compared with chemotherapy as a consolidation (5-year EFS: 68.4% 50.8%, = 0.26; 5-year OS: 65.9% 54.8%, = 0.45).
It could be concluded that can be detected in approximately 10% of pediatric AML cases. It is predominantly present in unfavorable cytogenetic subtypes and predicts adverse outcomes. Whether pediatric patients with AML can benefit from HSCT in CR1 needs to be researched further.
高嗜亲性病毒整合位点1（ ）表达是成人急性髓系白血病（AML）的一个独立预后因素。然而，关于 在儿童AML中的预后价值知之甚少。本研究旨在探讨 在来自中国七个中心的一大群接受统一治疗的儿童AML患者中的生物学及预后意义。
开发了一种诊断检测方法，使用单重实时定量聚合酶链反应来确定421例来自中国南方七个中心的新诊断的14岁以下儿童AML患者的相对 表达。所有患者均采用统一方案治疗，但仅383例患者接受了治疗反应评估。生存数据纳入后续分析（ 组n = 35， 组n = 348）。
在所有421例儿童AML患者中，9.0%检测到 。 主要见于急性巨核细胞白血病（FAB M7）、 重排和不良细胞遗传学异常，而与t（8；21）、inv（16）/t（16；16）、 、 或 突变相互排斥。在单变量Cox回归分析中， 具有显著不良的5年无事件生存期（EFS）和总生存期（OS）[风险比（HR）分别为1.821和2.401， 分别为0.036和0.005]。在多变量Cox回归分析中， 是OS的独立预后因素（HR = 2.447， = 0.015），但不是EFS的独立预后因素（HR = 1.556，p = 0.174）。此外， 是 重排患者OS和EFS的独立不良预测因素（单变量分析：HR分别为9.921和7.253，均 < 0.001；多变量分析：HR分别为7.186和7.315， 分别为0.005和0.001）。与作为巩固治疗的化疗相比，首次完全缓解（CR1）时进行造血干细胞移植（HSCT）使 患者有生存获益的趋势（5年EFS：68.4%对50.8%， = 0.26；5年OS：65.9%对54.8%， = 0.45）。
可以得出结论，约10%的儿童AML病例可检测到 。它主要存在于不良细胞遗传学亚型中，并预测不良预后。 AML儿童患者在CR1时是否能从HSCT中获益需要进一步研究。
